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EF-hand domains of MCFD2 mediate interactions with both LMAN1 and coagulation factor V or VIII
其他题名EF-hand domains of MCFD2 mediate interactions with both LMAN1 and coagulation factor V or VIII
ZHENG CHUNLEI; LIU HUIHUI; Zhou JH(周佳海); ZHANG BIN
2010
发表期刊Blood
ISSN0006-4971
卷号115期号:5页码:1081-1087
摘要Combined deficiency of factor V and factor VIII (F5F8D) is a bleeding disorder caused by mutations in either LMAN1 or MCFD2. LMAN1 (ERGIC-53) and MCFD2 form a Ca2+-dependent cargo receptor that cycles between the endoplasmic reticulum (ER) and the ER-Golgi intermediate compartment for efficient transport of FV/FVIII from the ER to the Golgi. Here we show that the C-terminal EF-hand domains are both necessary and sufficient for MCFD2 to interact with LMAN1. MCFD2 with a deletion of the entire N-terminal non-EF hand region still retains the LMAN1-binding function. Deletions that disrupt core structure of the EF-hand domains abolish LMAN1 binding. Circular dichroism spectroscopy studies on missense mutations localized to different structural elements of the EF-hand domains suggest that Ca2+-induced folding is important for LMAN1 interaction. The EF-hand domains also mediate the interaction with FV and FVIII. However, mutations in MCFD2 that disrupt the tertiary structure and abolish LMAN1 binding still retain the FV/FVIII binding activities, suggesting that this interaction is independent of Ca2+-induced folding of the protein. Our results suggest that the EF-hand domains of MCFD2 contain separate binding sites for LMAN1 and FV/FVIII that are essential for cargo receptor formation and cargo loading in the ER. (Blood. 2010;115:1081-1087)
学科领域生命有机化学
部门归属美国Cleveland Clin Fdn; 中国科学院上海有机化学研究所
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收录类别SCI
语种英语
WOS记录号WOS:000274287500022
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被引频次:12[WOS]   [WOS记录]     [WOS相关记录]
文献类型期刊论文
条目标识符http://ir.sioc.ac.cn/handle/331003/27801
专题上海有机化学研究所
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GB/T 7714
ZHENG CHUNLEI,LIU HUIHUI,Zhou JH,et al. EF-hand domains of MCFD2 mediate interactions with both LMAN1 and coagulation factor V or VIII[J]. Blood,2010,115(5):1081-1087.
APA ZHENG CHUNLEI,LIU HUIHUI,周佳海,&ZHANG BIN.(2010).EF-hand domains of MCFD2 mediate interactions with both LMAN1 and coagulation factor V or VIII.Blood,115(5),1081-1087.
MLA ZHENG CHUNLEI,et al."EF-hand domains of MCFD2 mediate interactions with both LMAN1 and coagulation factor V or VIII".Blood 115.5(2010):1081-1087.
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